Progressive facial atrophy

In the literature, this disease is known under two terms: half progressive facial atrophy (hemiatrophia faciei progressiva) and bilateral progressive facial atrophy (atrophia faciei progressiva bilateralis).

In addition, half and cross atrophy of the face and body can be observed.

[1], [2], [3], [4],

Causes of the progressive facial atrophy

It is suggested that the disease may be due to trauma to the skull or face, general or local infection, syphilis, syringomyelia, V or VII damage to the cranial nerves, extirpation or injury of the cervical sympathetic trunk, etc. Some authors allow for the possibility of hemiatrophy of a person combined with hemiatrophy bodies on the basis of dystrophy in the diencephalic parts of the autonomic nervous system.

There are cases of hemiatrophy after epidemic encephalitis, as well as in pulmonary tuberculosis, which has seized the cervical sympathetic trunk.

According to reports, progressive facial atrophy in the vast majority of cases is a syndrome of various diseases in which the pathological process involves the autonomic nervous system at different levels. Obviously, trauma and other factors are only an impetus to the development of these serious neuro-dystrophic phenomena.

[5], [6], [7], [8], [9], [10], [11], [12]

Symptoms of the progressive facial atrophy

Patients usually complain that the sick half of the face is less healthy; the difference in the volume of the facial part of the skull and soft tissues is gradually increasing; on the side of the lesion, the skin is darkish-slate, thinned, collected during a smile into a multitude of folds.

Sometimes patients notice tingling pain in the area of the affected cheek or in the entire half of the face, tearing of the eye on the affected side, especially in the cold, in the wind, and the difference in cheeks color, especially noticeable in the cold.

With pronounced hemiatrophy, one gets the impression that one half of the face belongs to a person who is exhausted by starvation or cancer intoxication, and the second to a healthy one. The skin of the affected side is yellowish-gray or brownish in color, not covered in blush. The ocular cleft is enlarged due to the westernization of the lower eyelid.

When pressing on the supraorbital, infraorbital and chin holes there is pain.

The corneal reflex is lowered, but the pupils are evenly dilated and react equally to light.

Thin skin feels like parchment; Atrophy extends to subcutaneous fat, chewing and temporal muscles, bone tissue (jaws, cheekbone and zygomatic arch).

The chin is displaced to the sore side, as the size of the body and the branches of the lower jaw are reduced, this is especially pronounced in patients suffering from hemiatrophic faces from childhood; half of the nose is also reduced, the auricle is wrinkled.

In some cases, facial hemiaphrophy is combined with atrophy of the same half of the trunk, and sometimes - with atrophy of the opposite side of the trunk (hemiatrophia cruciata), with unilateral scleroderma or excessive pigmentation in the skin, growth disorders or depigmentation of hair, hemiatrophy of the tongue, soft palate and alveolar processes , carious disease and loss of teeth, a violation of sweating.

Having reached one degree or another, facial hemiaphrophy is suspended, stabilized and does not progress further.

Clinical and physiological examinations of this contingent of patients showed that with all forms of progressive atrophy of the face, there are some more pronounced violations of the function of the autonomic nervous system.

In patients with unilateral dystrophy of the face, as a rule, asymmetry of indicators of electrical potentials and skin temperature with their predominance on the side of the lesion is revealed.

In most cases there is a decrease in the oscillographic index and spasm of the capillaries on the diseased side, which indicates a predominance of the tone of the sympathetic nervous system.

Almost all patients on electroencephalograms reveal changes characteristic of the defeat of hypothalamic-mesencephalic brain formations. With electromyographic studies, almost always detect changes in electrical muscle activity on the side of the dystrophy, including where clinically observed atrophic manifestations in tissues.

Based on the complex of data from clinical and physiological studies, LA. Shurinok distinguishes two stages of facial atrophy - progressive and stationary.

Diagnostics of the progressive facial atrophy

Facial hemiaphrophy should be differentiated with asymmetry in case of congenital (non-progressive) hypoplasia of the face, half of face hypertrophy, as well as muscular torticollis, focal scleroderma, tissue atrophy in lipodystrophies and dermatomyositis. The last diseases are considered in courses of general orthopedics and dermatology.

[13], [14], [15], [16], [17], [18]

Treatment of the progressive facial atrophy

Surgical methods for treating progressive facial atrophy are only permissible (!) After the process is stopped or slowed down, that is, in the second complete stage of the process. For this purpose, a complex medical and physiotherapeutic treatment is recommended in combination with a vago-sympathetic blockade, and sometimes a blockage of the cervicothoracic node.

To improve tissue metabolism should be prescribed vitamins (thiamine, pyridoxine, cyanocobalamin, tocopherol acetate), aloe, vitreous body or lidazu for 20-30 days. To stimulate metabolism in muscle tissue, intramuscularly injected with ATP 1-2 ml for 30 days. Thiamine promotes normalization of carbohydrate metabolism, as a result of which the amount of ATP (formed by oxidative phosphorylation going into the mitochondria) increases. Cyanocobalamin, nerobol, retabolil promote the normalization of protein metabolism.

To influence the central and peripheral parts of the autonomic nervous system (NNS), electrophoresis is combined on the cervical sympathetic nodes, galvanic collar, endonasal electrophoresis with 2% calcium chloride or diphenhydramine (7-10 sessions), UHF on the hypothalamic region (6 -7 sessions) and a galvanic half mask with lidase (№ 7-8).

It is necessary to exclude foci of irrigation emanating from the liver, stomach, pelvic organs, etc.

With an increased tone of the sympathetic and simultaneous weakness of the parasympathetic parts of the nervous system, it is recommended to combine sympatholytic and cholinomimetic drugs, taking into account the level of damage: when central vegetative structures are affected, central adrenolytics (aminazine, oxazil, reserpine, etc.) are prescribed: ganglions pachycarpine, hexonium, pentamine, gangleron, etc.). When involved in the process and peripheral and central departments of the NNS use antispasmodics such as papaverine, dibazol, euphilin, platyfilin, kellin, spasmolitin, nicotinic acid.

Sympathetic tone is reduced by limiting the diet of proteins and fats; To enhance parasympathetic influence, acetylcholine, carbacholin, and anticholinesterase substances (for example, proserin, oxamazine, mestinone) and antihistamines (diphenhydramine, pipolfen, suprastin) are prescribed. In addition, food rich in carbohydrates, mountain or sea climate is low, carbonates (37 ° C) and other means and methods prescribed by neuropathologists (LA Shurinok, 1975).

As a result of conservative preoperative treatment, the process stabilizes, although atrophy, as a rule, is externally expressed as before.

On myograms of the muscles of the face, there is an increase in their bioelectrical activity, a decrease or even disappearance of the asymmetry of the indices of the vegetative nervous system, a reduction in the number of electric potentials of the facial skin in a number of cases (initial forms of the disease), and the disappearance of thermotopography violations of the skin.

Methods of surgical treatment of progressive facial atrophy

The main methods of surgical treatment of facial atrophy include the following.

1. Injections of paraffin under the skin of atrophied cheeks. Because of the cases of thrombosis and embolism of the vessels, surgeons are not currently using this method.
2. Podsadka subcutaneous tissue (due to its gradual and uneven wrinkling also found no widespread use).
3. The introduction of plastic explants, ensuring the elimination of asymmetry in the face at rest, but at the same time immobilizing the patient side and excluding the symmetry of the smile. Do not satisfy patients and the rigidity of plastic, located in those places that are characterized by softness and compliance. In this respect, the introduction of porous plastics is more promising, but there are no convincing reports of the results of their application in the literature. It is also recommended to use explants made of silicone, which possesses biological inertness and stable elasticity.
4. Podsadka under the skin of the crushed cartilage and connective tissue base filatah stalk, which has almost the same drawbacks: stiffness (cartilage), the ability to immobilize the face (cartilage, stem).
5. Podsadka deepidermizirovannogo and devoid of subcutaneous tissue of the skin flap or the belly shell of the testis of a bull according to the methods of Yu.I. Vernadsky.

Correction of the face contour according to Yu.I. Vernadsky

In the submandibular region a cut is made, through which, with the help of large curved blunt-edged Cooper scissors or a special digger with a long handle, exfoliate the skin, previously "elevated" with 0.25% Novocain solution.

Dampening and pressing the outside of the formed pocket, on the front surface of the abdomen under local anesthesia outline the outline of the future transplant according to a previously prepared plastic template. In the outlined area (before taking the transplant), the skin is de-epidermalized, and then the flap is removed, trying not to seize the subcutaneous tissue.

Taking a flap of plastic threads (holders), thread their ends in the eye 3-4 straight thick ("gypsy") needles, through which stretch the ends of the holders in the subcutaneous wound on the face, and then from the upper and lateral arches, the wounds outward and knotted on small iodoform rollers. Thus, the skin graft appears to be stretched across the entire subcutaneous wound surface. Due to the fact that the graft on both sides has a wound surface, it grows to the skin and subcutaneous tissues inside the wound pocket.

In places of greatest deviation, the cheeks are folded together or stacked in three layers by sewing to the main flap an additional "blotch" -displication. The cosmetic effect of this technique is quite high: the asymmetry of the face is eliminated; the mobility of the affected half of the face, although it decreases, is not completely paralyzed.

During and after surgery, there are usually no complications (unless an infection leading to the rejection of the graft or explant is attached). However, over time, some atrophy of the planted skin (or other biological material) occurs and it is necessary to add a new layer of it. In some patients, after the insertion of de-epidermis autistic skin, gradually increasing sebaceous cysts develop. In these cases, it is recommended to puncture the skin with a thick injection needle over the place of accumulation of fat (in 2-3 places) and squeeze it out through punctures. Then, the empty cavity is washed with 95% ethyl alcohol to induce denaturation of the activated cells of the sebaceous glands; part of the alcohol is left in the cavity under a pressure bandage, imposed for 3-4 days.

To avoid the formation of sebaceous cysts (athere) and additional trauma, it is advisable to use the belly of the bull testis instead of the autoderm, which is perforated with a scalpel in staggered order and injected under the skin of the affected area of the face (in the same way as the autoderm).

[19]

Correction of the contour of the face by the method of A.T. Titova and N.I. Yarchuk

The contour plastic is made with an allogeneic preserved broad fascia of the thigh, placing it in one or two layers or harmonizing it (corrugating it) if a considerable amount of plastic material is required.

A pressure bandage on the face is applied for 2.5-3 weeks.

2-3 days after the operation, the fluctuation in the area of transplantation is determined, not due to accumulation of fluid under the skin, but to edema of the fascial graft and aseptic inflammation of the wound.

To reduce the edema after the operation, the cold is applied to the transplant area within 3 days, and dimedrol is administered inside to 0.05 g 3 times a day for 5-7 days.

Postoperative swelling of the graft is dangerous in those cases where the incision for the formation of the bed and the insertion of the fascia is located directly above the transplant region. In this case, excessive tension can occur at the edges of the wound, leading to their divergence and loss of part of the fascia. To prevent this complication it is necessary that the cuts of the skin are located outside the transplanting area, and if it does occur, then in the early period one can confine oneself to removing a part of the fascial graft, and apply secondary seams to the wound.

When joining the infection and the development of inflammation in the wound, the entire graft should be removed.

Despite extensive tissue detachment during transplantation of the fascia, subcutaneous hematomas and intradermal hemorrhages are extremely rare, which can be explained to some extent by the hemostatic action of the fascial tissue. The greatest risk of formation of hematomas exists when the severe deformations of the side of the face are eliminated. Extensive detachment of tissues through the incision in front of the auricle creates a prerequisite for the accumulation of blood in the lower, closed section of the formed bed. If there is a suspicion of the formation of a hematoma, it is recommended to create an outflow in the lower part of the wound.

Complications

The most severe complication is the suppuration of the surgical wound that occurs when the transplant or the receiving bed is infected. For its prevention, it is necessary to strictly comply with the requirements of asepsis when preparing fascial grafts and during their transplantation, trying not to damage the mucous membrane of the oral cavity when forming a bed in the cheek and lip area.

The emergence of an operative wound message with the oral cavity during surgery is a contraindication for transplantation of the fascia. A white membrane, etc. Repeated intervention is permissible only after several months.

Given that the subcutaneous fatty tissue of the foot of the human foot (the thickness of which is from 5 to 25 mm), as well as the dermis of the foot, differ sharply from the fiber and derma of other areas, and also that they are very strong, dense, elastic, possess low antigenic properties, NE Selskii et al (1991) recommend this allo-material for contour plasty of the face.It applied it in 21 patients, the authors noted suppuration and rejection of the transplant in 3. It is obviously necessary to continue studying the immediate and long-term results of using this plaque matic material t. K., unlike the skin of other portions deepitelizirovannoy, plantar skin devoid of sweat and sebaceous glands, which is very important (in terms of prevention kistoobrazovanie).

[20], [21]